Primary cutaneous interdigitating dendritic cell sarcoma (IDCS): Report of a new case and literature review.

Interdigitating dendritic cell sarcoma is a very rare entity in the spectrum of histiocytic and dendritic cell neoplasms that mostly occurs in lymph nodes, generally presenting as solitary lymphadenopathy, but may affect every organ. Among extra nodal sites, cutaneous interdigitating dendritic cell sarcoma is exceedingly rare; to date, only 9 cases have been described in English literature. The mean age at diagnosis was 60 years, with a male-female ratio of 1,5 to 1; clinically, two different modalities of skin presentation have been reported: solitary, represented by a single red-brownish nodular lesion, or diffuse, characterized by multiple nodular lesions in one or more body districts. The extreme rarity of this sarcoma and its morphological similarity to other poorly differentiated tumors may lead to a delay in diagnosis; in particular, cutaneous localization may be difficult to differentiate from follicular dendritic cell sarcoma, Langerhans cell sarcoma, poorly differentiated squamous cell carcinoma and more generally sarcomatoid carcinoma, atypical fibroxanthoma, malignant melanoma and several sarcomas. Immunohistochemistry plays an important role in identifying this rare entity and formulating a correct histological diagnosis, fundamental requirement for choosing the best therapeutic approach. We report herein a further case of an 81-year-old Caucasian woman who presented to the Dermatology Department to remove an asymptomatic skin papule in the left temporal region, clinically diagnosed as dermatofibroma. The overall pathological and immunohistochemical features supported the diagnosis of a malignant dendritic cell tumor, consistent of interdigitating dendritic cell sarcoma.

An unusual case of extra-nodal interdigitating dendritic cell neoplasm: sarcoma or not?

INTRODUCTION: Interdigitating dendritic cell sarcoma (IDCS) represents an extremely rare neoplasm frequently originating from T-cell- associated dendritic antigen-presenting cells normally populating the paracortex of lymph nodes. Diagnosis is challenging due to the rarity of this neoplasm and the overlapping features with the other primary and metastatic spindle cell neoplasm, even more, when localized in an extra-nodal site. PATIENTS AND METHODS: Herein we report a case of 48 Years old woman with a six-month history of centimetric, quiet painful mass of the philtrum without other significant comorbidity. RESULTS: Histological report showed a proliferation of quiet bland spindle to plump cell, scattered multinucleated giant cell in a subtle background of lymphocytes. IHC study displays only positivity for S-100 and fine, granular scattered cytoplasmatic stain for CD68; all dendritic IHC markers were negative. Morphological and immunohistochemical analyses were consistent with extra-nodal Interdigitating Dendritic Cell Neoplasm. CONCLUSION: Interdigitating Dendritic Cell Sarcoma is a rare and challenging entity with a variable prognosis. We present a case of extra-nodal IDCS whit low worrisome histological features, emphasizing the need for further efforts to better definitely this rare neoplasm ad its potential for malignancy.

A rare case of cutaneous interdigitating dendritic cell sarcoma.

Interdigitating dendritic cell sarcomas (IDCSs) are aggressive tumors of dendritic cells, often presenting with lymphadenopathy. Fewer than 10 cases of primary cutaneous IDCS have been reported. Histopathologically, IDCS presents as atypical spindle cells with irregular nuclei, and therefore can be difficult to distinguish from melanoma, follicular dendritic cell sarcoma, and Langerhans cell tumors by H&E examination alone. We report a unique case of a man with cutaneous IDCS that was initially misdiagnosed as melanoma. Having previously undergone an excision of a reported "melanoma" on the neck, he presented with a new growth on the cheek. Histopathologic findings revealed an atypical dermal lymphohistiocytic infiltrate around vessels and cells forming nests along the dermal-epidermal junction. Immunohistochemical stains were strongly positive for S100, fascin, and lysozyme; on the other hand, CD1a, langerin, CD21, CD23, and SOX10 were negative. These immunohistochemical findings were consistent with IDCS, and the patient's prior biopsy specimen was then revisited. Similar staining revealed that lesion also to be a cutaneous IDCS. Follow-up imaging with PET scan was negative for metastases, supporting the diagnosis of primary cutaneous IDCS. Our findings contribute to the limited literature on cutaneous IDCS and highlight a potential pitfall in its diagnosis because of overlapping histopathologic features with melanoma.

Interdigitating dendritic cell sarcoma: analysis of two original extra-nodal cases and review of literature.

Interdigitating dendritic cell sarcoma (IDCS) is a rare, highly malignant tumor with a poor prognosis, and current knowledge of this tumor is limited. It is reported that lymph nodes are the primary localization sites. However, in recent years, many primary IDCS have also been reported in the extra-nodal sites, which undoubtedly increases the difficulty of diagnosis. There are very few reports that systematically analyze the clinicopathologic features of IDCS. Here we described two cases of extra-nodal IDCS and reviewed the literature of 44 other published cases of extra-nodal IDCS. Thus, the clinical symptoms, pathological diagnosis, and therapeutic effects of 46 cases of extra-nodal IDCS were summarized in detail. Considering the paucity of available data with regard to IDCS, a thorough and detailed summary would help to better diagnose and treat this neoplasm.

[Clinicopathological characteristics and differential diagnosis of interdigitating dendritic cell sarcoma].

Objective: To analyze the clinicopathological features and differential diagnosis of interdigitating dendritic cell sarcoma (IDCS). Methods: The clinical pathological features of 7 IDCS were analyzed. Among them, the follow-up results of 6 cases were available. Results: Among the 7 IDCS patients, 4 cases were male and 3 were female. The age of the patients ranged from 26 to 69 years.Three cases were originated from lymph nodes and 4 cases were originated from skin, stomach, adrenal gland and mesentery, respectively. Microscopically, the tumor cells presented as fascicular and storiform proliferation and infiltrated by lymphocytes. The tumor cells were short-spindle or ovoid, with indistinct border of cytoplasm. The immunohistochemistry results showed that tumor cells were S-100, Vim, CD68 and CD163 positive, and AE1/AE3, EMA, CD117, CD34, Desmin, SMA, CD1α, CD21, CD23, CD35, HMB45, Melan-A, MelanPan and ALK negative.The BRAF mutation and clonal rearrangement of T and B cells were not detected. Among the follow-up period of 7 IDCS patients, 3 occurred disease progressions. Conclusions: IDCS is extremely rare with unique pathological features, and its lesion is not limited to the lymph node. The IDCS patients with extensive lesions may have worse prognose. The differential diagnosis of IDCS includes other histiocytic and dendritic cell neoplasms, malignant melanoma and soft tissue neoplasms.

Interdigitating dendritic cell sarcoma of the spleen with hepatic failure after chemotherapy: A case report.

RATIONALE: Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare disease originating from dendritic cells (DCs). There are few cases report interdigitating dendritic cell sarcoma of spleen along with their pathological characteristics and treatment. PATIENT CONCERNS: Here we report a case of IDCS in 53-year-old female who presented spleen enlargement and thrombocytopenia. DIAGNOSES: The patient underwent surgical resection of spleen, and the pathology confirmed IDCS. INTERVENTIONS: She received surgical resection of spleen and one cycle of chemotherapy (ABVD with ifosfamide and oxaliplatin) after surgery. OUTCOMES: She died of severe hepatic failure caused by chemotherapy. DISCUSSION: IDCS is a rare disease with insufficient treatment guidelines. We adopted chemotherapy of ABVD with ifosfamide and oxaliplatin which showed no improvement but led to life-threatening liver damage.

Interdigitating dendritic cell sarcoma: Clinicopathologic study of 8 cases with review of the literature.

To investigate the clinicopathologic features and differential diagnoses of interdigitating dendritic cell sarcoma (IDCS), the clinical, morphological and immunohistochemical features of eight cases of IDCS were collected and analyzed. Three patients were males and five were females, the mean age and the median age were 56.5 years and 57 years respectively. Clinically, the majority of cases involved lymph nodes. Microscopically, neoplastic cells were spindle or ovoid, forming fascicles or whorls. Every case had active mitosis figures. Immunohistochemically, these neoplastic cells were consistently positive for S100, but negative for CD21 and specific B-cell and T-cell associated antigens. Follow-up results were available in 7 cases, of which 5 cases of localized lesions survived, 2 cases died of organ involvement. Interdigitating dendritic cell sarcoma is an extremely rare neoplasm, with inferior prognosis and without standard treatment regimen. IDCS has similar but unique clinicopathologic features and the differential diagnoses include other histiocytic and dendritic cell neoplasms and malignant melanoma.

Diffuse lesion and necrosis tied to poorer prognosis of interdigitating dendritic cell sarcoma: cases report and a pooled analysis.

Interdigitating dendritic cell sarcoma is a neoplastic proliferation of interdigitating dendritic cells and no therapeutic consensus exists. This study aimed to investigate the prognostic impacts of tumor lesion, cellular atypia, mitosis and necrosis on the interdigitating dendritic cell sarcoma. Case reports and pooled analyses were designed to explore the relationships. One case was a 40-years old man with localized lesion, moderate to notable cellular atypia, 30 mitoses per 10 high-power fields and no necrosis and the progression-free survival was longer than 20 months. The other case was a 62-years old woman with diffuse lesion, notable cellular atypia, less than one mitosis per 10 high-power fields and diffuse necrosis and the progression-free survival was shorter than 1 month. Cellular atypia and mitosis had not any relationship with survival. Compared with localized lesion, diffuse lesion presented a 2.92-fold risk of progression (HR = 2.92, 95% CI 1.01, 8.51) and an 8.79-fold risk of death (HR = 8.79, 95% CI 1.86, 41.64). Diffuse necrosis presented a 4.39-fold higher progression risk (HR = 5.39, 95% CI 1.78, 16.29) and a 5.37-fold higher death risk (HR = 6.37, 95% CI 1.46, 27.86) than focal or no necrosis. Diffuse lesion and diffuse necrosis were indicators of poorer prognosis and the clinical application should be warranted in further studies.

Primary cutaneous interdigitating dendritic cell sarcoma is a morphologic and phenotypic simulator of poorly differentiated metastatic melanoma: A report of 2 cases and review of the literature.

Interdigitating dendritic cell sarcoma (IDS) is a rare form of hematologic malignancy associated with an aggressive clinical course. Only 4 prior cases have been described as originating in the skin. We encountered two male patients ages 47 and 61years of age who presented with solitary cutaneous neoplasms diagnosed as IDS. Histologic exam showed a coalescing nested and multinodular proliferation of large pleomorphic epithelioid cells. In one case an initial diagnosis of melanoma was rendered. A recurrence 8months later was then interpreted as a primary cutaneous IDS. This patient died of widespread metastatic disease within 2years from his initial surgery. The other patient has recently undergone wide excision and radiation without any recurrence or metastatic disease during this short follow up time period. Both patients had a tumor exhibiting the same phenotypic profile comprising leukocyte common antigen, SOX10, S100, CD68, and CD163 positivity. In reviewing the 4 other reported cases, there is a similar older male predominance (mean age of 58years) although women affected were significantly younger (mean age of 28years); there was a predilection for the proximal extremities and the face. Patients treated with excision only developed recurrent disease with one patient subsequently dying of metastatic disease. Primary cutaneous IDS is a highly aggressive hematologic malignancy that has many overlapping features with poorly differentiated epithelioid and spindle cell melanoma including SOX10 staining. An aggressive treatment protocol at the beginning could optimize patient survival.

Head and Neck Extranodal Interdigitating Dendritic Cell Sarcoma: Case Report and Review of the Literature.

Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare neoplasm originating from professional antigen presenting cells normally located in the T zone of the lymph node. The purpose of this report was to describe the first case of the IDCS of the submandibular gland and perform a review of the literature of head and neck IDCS. We present a case of an 81-year-old man with a 5 months history of slowly enlarging painless mass in right submandibular region. Fine needle aspiration cytology was suggestive of squamous cell carcinoma. The patient underwent surgical resection of the right submandibular gland and neck dissection. A malignant spindle cell proliferation involving the submandibular gland and colonizing one laterocervical lymph node was found. Morphology and immunophenotype prompted a differential diagnosis of a metastatic spindle cell melanoma versus an IDCS. Transmission electron microscopy was performed and supported a diagnosis of IDCS. The diagnosis of IDCS is a challenging task and may require a large array of techniques.

Intracranial Interdigitating Dendritic Cell Sarcoma: First Case Report.

BACKGROUND AND IMPORTANCE: This is the first report of a primarily intracranial interdigitating dendritic cell sarcoma (IDCS). CLINICAL PRESENTATION: A 39-year-old patient with right hemiparesis underwent complete resection of a large parafalcine tumor with subsequent complete recovery of neurological symptoms. Histologically, the tumor was diagnosed as IDCS. Extensive staging did not reveal any extracranial manifestation of this disease. After 1.5 years, the patient remains recurrence free and is being observed closely. CONCLUSION: IDCS are exceedingly rare tumors and so far have not been found intracranially. On the basis of the limited experience with extracranial occurrence, this tumor is best managed by complete resection and careful oncological observation. ABBREVIATIONS: FDCS, follicular dendritic cell sarcomaIDCS, interdigitating dendritic cell sarcomaRTU, ready-to-use kit.

Complete Remission in Systemic Skin Interdigitating Dendritic Cell Sarcoma after ABVD Chemotherapy.

Interdigitating dendritic cell sarcoma (IDCS) is a rare and aggressive neoplasm that is thought to arise from dendritic cells. This disease usually involves the lymph nodes and, rarely, extra-nodal sites. We report a 62-year-old man presenting skin nodules in the head, body, and extremities, as well as bone marrow involvement. Morphologic analysis of a biopsied specimen from the skin lesion was consistent with IDCS. Immunohistochemical staining demonstrated that the tumor cells were positive for IDCS-associated antigens such as CD4, CD45, CD68 (KP-1), and S-100 protein. Complete remission was achieved by treatment with 6 cycles of ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) chemotherapy. Although the optimal treatment of IDSC remains unknown, the experience in the current case supports the notion that ABVD chemotherapy may be effective for IDCS, and further extends this idea to rare patients presenting multiple skin lesions.

Spontaneous regression of interdigitating dendritic sarcoma in a patient with concurrent small lymphocytic lymphoma.

Interdigitating dendritic cell sarcoma (IDCS) is a rare tumour; only seven cases of IDCS associated with chronic lymphocytic leucaemia/small lymphocytic lymphoma (CLL/SLL) have been reported. We present the case of a 60-year-old man who presented with fevers, night sweats and significant unintentional weight loss. Investigations led to a diagnosis of synchronous SLL and IDCS. Subsequent fluorodeoxyglucose (FDG) positron emission tomography CT (PET-CT) imaging revealed an unusual clinical course with spontaneously resolving highly metabolic lesions. After 42 months of follow-up, the patient remains free of clinical symptoms and evidence of IDCS.

Challenges in the differential diagnosis of interdigitating dendritic cell sarcoma of intraparotid lymph node vs. metastatic malignant melanoma with unknown primary site.

Interdigitating dendritic cell sarcoma (IDCS) is an uncommon form of malignant histiocytosis affecting dendritic cells. The parotid gland more frequently than other salivary glands has metastasis from extraparotid tumours, which in 80% of cases are melanomas and squamous cell carcinomas. Herein we report our case, a 64-year-old woman who presented with a short history of fluctuating in size swelling below her right ear. Ultrasound scan showed a loculated cystic lesion extending in the parotid parenchyma. Fine needle aspiration (FNA) revealed appearances that were highly suspicious of malignancy, therefore MRI scan was arranged, and parotidectomy planned. The histology of tumour was a malignant spindle cell neoplasm, with immunohistochemical features highly suggestive of metastatic malignant melanoma with divergent differentiation. The challenges in the differential diagnosis of IDCS of intraparotid lymph node vs. metastatic malignant melanoma with unknown primary tumour are described here. The rarity of this neoplasm figures highlights the importance of describing all new cases putting special emphasis on the steps to be taken in order to shorten the diagnosis, management and treatment process.

Interdigitating dendritic cell sarcoma.

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare dendritic cell tumor with slightly more than 100 cases reported in the English literature. This report discusses a case of localized IDCS involving cervical lymph nodes and provides a literature review of clinicopathologic aspects and treatment outcomes.

Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.

Both interdigitating cell sarcoma (IDCS) and follicular dendritic cell sarcoma (FDCS) are rare neoplasms derived from dendritic cells in lymphoid organs. IDCS is defined as a neoplastic proliferation of spindle-shaped to ovoid cells with phenotypic features similar to those of IDCs. FDCS is a malignant neoplasm derived from FDCs that possess and present antigens to B cells in the follicular (germinal) centers of lymphoid organs. They often occur in lymph nodes, although they can also arise at extranodal sites. In this review, we have highlighted the morphological and immunohistochemical properties of these neoplasms, which could help in unequivocal and accurate diagnosis.

Interdigitating dendritic cell sarcoma presenting simultaneously with acute myelomonocytic leukemia: report of a rare case and literature review.

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare tumor derived from interdigitating dendritic cells. We report the first case of a 64-year-old Chinese woman who was diagnosed with simultaneous IDCS and acute myelomonocytic leukemia (AML-M4). The patient had undergone chemotherapy for breast cancer 6 years previously. Based on the laboratory results, both the IDCS and the AML-M4 in this patient were determined to be of myelogenous origination. Furthermore, a review of 62 IDCS cases (Medline database, key word: IDCS) reported to date revealed that as many as 17 % of the patients had malignant disease and received radiotherapy and/or chemotherapy prior to developing IDCS, and that this group of patients showed worse prognosis compared with counterparts. The patient in the present report showed poor response to four cycles of sequential chemotherapy, and died 6 months after the initial diagnosis.